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Background
A vasculitis is an inflammation of a blood vessel. These blood vessels may be as small as capillaries to as large as the aorta. Surprisingly, many vasculitides affect only a certain caliber of vessels, although there are conditions which affect all. Because any vessel can be affected the diagnosis is often not suspected clinically and requires a biopsy to establish the diagnosis.
ANCA
Behcet's Disease
Churg-Strauss Syndrome (Allergic Granulomatosis)
Erythema Elevatum Diutinum
Erythema Induratum
Granuloma faciale
Henoch-Schonlein Purpura
Kawasaki's Disease
Leukocytoclastic Vasculitis
Microscopic Polyarteritis (Microscopic polyangiitis)
Polyarteritis Nodosa
Pustular vasculitis of the dorsal hands
Takayasu's Arteritis
Temporal Arteritis (Giant Cell Arteritis)
Wegener's GranulomatosisOUTLINE
CLINICAL VARIANTS CHARACTERIZATION GENERAL
From the *Divisions of Dermatology and Dermatopathology, Albany Medical College, MC-81, Albany, NY 12208; and daggerDepartment of Dermatology, Saiseikai Central Hospital, 1-4-17 Mita, Minato-Ku, Tokyo 108-0073, Japan.
Most biopsies of cutaneous vasculitis will exhibit a small vessel neutrophilic vasculitis [leukocytoclastic vasculitis (LCV)] that is associated with immune complexes on direct immunofluorescence examination or, less commonly, antineutrophilic cytoplasmic antibodies (ANCA) by indirect immunofluorescence testing.
Is in uncommon for skin biopsy to reveal solely a neutrophilic arteritis signifying the presence of cutaneous polyarteritis nodosa or, if accompanied by significant lobular panniculitis, nodular vasculitis/erythema induratum. In other cases, cutaneous vascular damage (fibrinoid necrosis, muscular vessel wall disruption, or endarteritis obliterans) will be mediated by a nonneutrophilic inflammatory infiltrate. Eosinophilic vasculitis can be a primary (idiopathic) process that overlaps with hypereosinophilic syndrome, or it can be a secondary vasculitis associated with connective tissue disease or parasite infestation. Authentic cutaneous granulomatous vasculitis (versus vasculitis with extravascular granulomas) can represent a cutaneous manifestation of giant cell arteritis, an eruption secondary to systemic disease such as Crohn's disease or sarcoidosis, or a localized disorder, often a post-herpes zoster (HZ) phenomenon. Lymphocytic vasculitis is a histologic reaction pattern that correlates with broad clinical differential diagnosis, which includes connective tissue disease-mostly systemic lupus erythematosus (SLE), endothelial infection by Rickettsia and viruses, idiopathic lichenoid dermatoses such as perniosis or ulcerative necrotic Mucha-Habermann disease, and angiocentric cutaneous T-cell lymphomas.
Skin biopsy extending into the subcutis, identifying the dominant inflammatory cell and caliber of vessels affected, extravascular histologic clues such as presence of lichenoid dermatitis or panniculitis, and correlation with clinical data allows for accurate diagnosis of these uncommon vasculitic entities.
From the *Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, NY; and the daggerDepartment of Dermatology, Saiseikai Central Hospital, Tokyo, Japan.
A broad and diverse spectrum of vasculitic syndromes exists. These syndromes affect the skin with varying levels of associated systemic manifestations, running the gamut from a self-limited, localized, cutaneous phenomenon to rapidly progressive, multiorgan disease. The majority of cases of cutaneous vasculitis will show a neutrophilic small vessel vasculitis that can be either a primary (idiopathic) disorder (eg, cutaneous leukocytoclastic angiitis) or a secondary disorder that is associated with drugs, infection (eg, streptococcal infection, viral hepatitis), or underlying disease (eg, connective tissue disease, malignancy).
Biopsy is the gold standard for the diagnosis of cutaneous vasculitis and also necessary for the detection of cutaneous vascular immune complexes by direct immunofluorescence. Based on the type of vessel disrupted by inflammation (small and/or muscular), the distribution of vasculitis in the dermis and subcutis, and predominate inflammatory cell-type mediating vessel wall damage, a list of relevant differential diagnoses can be generated.
This histologic information coupled with extravascular findings such as tissue eosinophilia, tissue neutrophilia, and/or granulomas, plus pathophysiologic markers such as direct immunofluorescent examination for immune complexes and serologic evaluation for antineutrophil cytoplasmic antibodies allows for more accurate diagnosis of specific vasculitic entities.
Herein, we review both primary and secondary vasculitic syndromes that affect the skin and show a small vessel neutrophilic mediated vasculitis.
Cutaneous vasculitis: a review.Crowson AN, Mihm Jr MC, Magro CM.
Dermatology and Pathology, University of Oklahoma and, Regional Medical Laboratory, St. John Medical Center, Tulsa, OK, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA Department of Pathology Ohio State University, Columbus, OH, USA.
J Cutan Pathol 2003 Mar;30(3):161-173 Abstract quote As the skin is commonly involved in systemic vasculitic disorders as well as those hypersensitivity states whose expression is largely skin-confined, cutaneous vasculitic lesions offer a window to diagnosis and a ready source of accessible tissue for biopsy.
In this review, we discuss the pathologic manifestations of chronic vasculitic syndromes such as granuloma faciale and erythema elevatum diutinum; IgA-associated vasculitis including Henoch-Schonlein purpura; vasculitis seen in the setting of cryoglobulinemia and hypergammaglobulinemia of Waldenstrom, hereditary deficiencies of complement, and IgA deficiency; those leukocytoclastic vasculitides resulting from hypersensitivity reactions to drug, chemical and foodstuff ingestion; and those vasculitides seen in patients with systemic diseases such as polyarteritis nodosa, rheumatoid arthritis, mixed connective tissue disease, systemic lupus erythematosus, Sjogren's syndrome, relapsing polychondritis, Behcet's disease, Wegener's granulomatosis, and allergic granulomatosis of Churg and Strauss.
Cutaneous vasculitis.Fiorentino DF.
Department of Dermatology, Stanford University School of Medicine, CA 94305, USA.
J Am Acad Dermatol 2003 Mar;48(3):311-40 Abstract quote Vasculitis can range in severity from a self-limited single-organ disorder to a life-threatening disease with the prospect of multiple-organ failure. This condition presents many challenges to the physician, including classification and diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. The physician must not only be able to recognize vasculitis but also be able to provide a specific diagnosis (if possible) as well as recognize and treat any underlying etiologic condition.
Most diagnostic criteria are based on the size of vessel involvement, which often correlates with specific dermatologic findings. This may allow the dermatologist to provide an initial diagnosis and direct the medical evaluation.
This article reviews the classification and diagnosis of cutaneous vasculitic syndromes and current treatment options; it also presents a comprehensive approach to diagnosing and treating the patient with suspected cutaneous vasculitis.
VARIANTS GYRATE ERYTHEMA Leukocytoclastic vasculitis presenting as an erythema gyratum repens-like eruption on a patient with systemic lupus erythematosus.
Pique E, Palacios S, Santana Z.
Departments of Dermatology, Pathology, and Internal Medicine, Hospital General de Lanzarote, Arrecife de Lanzarote.
J Am Acad Dermatol 2002 Nov;47(5 Suppl):S254-6 Abstract quote Cutaneous leukocytoclastic vasculitis is a common finding among patients with systemic lupus erythematosus, although the clinical appearance of the lesions varies.
We report the case of a 38-year-old woman with systemic lupus erythematosus who had leukocytoclastic vasculitis with peculiar clinical morphologic features of the cutaneous lesions. They consisted of figured erythema closely resembling erythema gyratum repens.
We discuss the differential diagnosis with other annular eruptions.
LIVEDO RETICULARIS
- Livedo reticularis: an update.
Gibbs MB, English JC 3rd, Zirwas MJ.
Department of Dermatology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
J Am Acad Dermatol. 2005 Jun;52(6):1009-19. Abstract quote
Livedo reticularis (LR) is a well-known, relatively common physical finding consisting of macular, violaceous, connecting rings that form a netlike pattern (Fig 1). In most cases, it is a completely benign finding related to cold exposure. However, there are many potential causes (Table I), and this can make the evaluation of a patient presenting with this finding very difficult.
An excellent review of the topic by Fleischer and Resnick was published in 1990. We have endeavored to update the literature and provide clinicians with guidance regarding the evaluation and treatment of patients presenting with LR.LYMPHOCYTIC THROMBOPHILIC ARTERITIS
Skin and Cancer Foundation Australia, Sydney, New South Wales, Australia.
BACKGROUND: We encountered a distinct arteriolar histopathologic finding of lymphocytic vasculitis associated with a hyalinized fibrin ring in vessel lumina. Identical histologic findings have previously been described as macular arteritis.
OBSERVATIONS: We describe 5 women (mean age, 25 years; age range, 20-34 years) with persistent, slowly progressive, patchy and reticular hyperpigmentation associated with livedo racemosa affecting predominantly the lower limbs. In the biopsy samples, infiltration of muscular vessel wall by inflammatory cells, affecting small arteries of the dermosubcutaneous junction or superficial subcutis, was present. Of the infiltrate, 90% or more consisted of mononuclear cells, mainly lymphocytes with an admixture of histiocytes. Neutrophils and eosinophils were absent or scant. Inflammation was confined to the vicinity of the vessel and the immediate surrounding panniculus. A concentric fibrin ring involving the entire periphery of the lumina of affected vessels was present in all the patients. Laboratory investigation results revealed that 4 patients had antiphospholipid antibodies in their serum. One of these patients had a heterozygous mutation of the factor V Leiden gene.
Conclusion We term this arteritis lymphocytic thrombophilic arteritis to reflect the histologic features that combine lymphocytic vascular inflammation with changes representing a thrombophilic endovasculitis.SURAL NERVE
Clinicopathologic study of 43 patients with sural nerve vasculitis.Prayson RA, Sedlock DJ.
Hum Pathol. 2003 May;34(5):484-90. Abstract quote Vasculitis is a relatively uncommon finding in sural nerve biopsy specimens and is associated with significant morbidity. This study retrospectively reviewed the clinicopathologic features of 43 patients (44 sural nerve biopsy specimens) with sural nerve vasculitis, defined as infiltration of vessel walls by inflammatory cells.
These biopsy specimens were obtained over a 19-year period, during which 1503 nerve specimens were reviewed. The study group comprised 29 females and 14 males, ranging in age from 19 to 94 years (mean, 72.5 years) at the time of biopsy. The most frequent clinical presentations included paresthesias in 19 patients (61%), pain in 17 patients (57%), weakness in 10 patients (32%), and weight loss in 9 patients (29%).
Histologically, 26 of 44 biopsy specimens (59%) demonstrated necrotizing vasculitis. The remaining 18 biopsy specimens demonstrated a nonnecrotizing lymphocytic vasculitis. Eosinophils were identified in 4 biopsy specimens, intravascular thrombi in 10 (22%) specimens, and granulomatous inflammation in 1 specimen. In 39 biopsy specimens (89%), multiple vessels were involved by vasculitis. Epineurial vessels were the most common target of vasculitis, (n = 42; 95%). Evidence of vascular wall scarring, indicative of healed vasculitis, was observed in 13 biopsy specimens (30%). All biopsy specimens showed evidence of axonopathy, with mild axonal loss noted in 14 specimens (32%), moderate loss in 18 specimens (41%), and severe loss in 12 specimens (27%). Concomitant muscle biopsy was performed in 31 patients. Fifteen muscle biopsy specimens demonstrated evidence of vasculitis (48%), which was necrotizing in 11 cases.
All muscle biopsy specimens demonstrated features of neurogenic atrophy. Twenty-five out of 32 patients were known to have been treated with steroids and demonstrated some degree of clinical improvement.
In conclusion, sural nerve vasculitis is a relatively uncommon pathological finding (prevalence of 2.9% in this study). In most cases, multiple vessels were involved. Concomitant vasculitis was also identified in about half of the muscle biopsy specimens obtained at the time of nerve biopsy. In most patients, the vasculitis appeared to be at least partially responsive to immunosuppressive therapy.PROLONGED URTICARIA WITH PURPURA
National Skin Centre, National University Hospital, Singapore.
BACKGROUND: Biopsy specimens of lesions with clinical features of urticarial vasculitis often show a predominantly lymphocytic infiltrate with eosinophils and red blood cell extravasation. Only occasionally is a leukocytoclastic vasculitis encountered, confirming a diagnosis of urticarial vasculitis.
OBJECTIVE: The aim of this study was to assess the clinical presentation and histologic features of patients who meet the clinical criteria for urticarial vasculitis.
METHODS: Patients were recruited who had persistent urticarial lesions individually lasting longer than 24 hours, associated with at least 2 of 3 of the following: pain or tenderness; purpura or dusky changes; and resolution with hyperpigmentation. Patients were interviewed based on a standard questionnaire with regard to their symptoms. Blood tests and chest radiographs were performed to exclude systemic involvement and hypocomplementemia. Skin biopsy specimens were sent for histology and direct immunofluorescence.
RESULTS: Of 22 patients recruited, 19 (86.4%) showed a predominantly lymphocytic infiltrate on histology. Three cases (13.6%) had a neutrophil-predominant infiltrate associated with a leukocytoclastic vasculitis. Twenty (90.9%) had a superficial perivascular infiltrate, and two (9.1%) had a superficial and deep perivascular infiltrate. In all, 21 biopsy specimens (95.5%) showed inflammatory cells within dermal blood vessel walls, obscuring the vessel outline in some. Endothelial cell swelling was seen in 20 biopsy specimens (90.9%), erythrocyte extravasation in 17 (77.3%), nuclear dust in 5 (22.7%), and fibrin extravasation in 2 (9.1%). Multivariate analysis revealed the following features to be independently associated with neutrophil predominance: fulfillment of all 3 minor criteria for urticarial vasculitis-like lesions (P = .007); presence of fibrin on histology (P < .001); presence of nuclear dust on histology (P = .001); hypocomplementemia (P = .001); and anemia (P = .015). There was a trend toward lesions not clearing as readily in the neutrophil-predominant group (P = .071), even with two-modality treatment (P = .089).
LIMITATIONS: Serum immunoelectrophoresis was not done to exclude Schnitzler's syndrome. Electronmicroscopy and cytokine profiling were not performed.
CONCLUSION: Biopsy specimens of lesions with clinical features of urticarial vasculitis reveal that only a minority of patients has leukocytoclastic vasculitis. The majority has a lymphocyte-predominant histology, associated with varying numbers of eosinophils. We favor a lymphocytic vasculitis as a causative explanation in the lymphocyte-predominant group.
HISTOPATHOLOGICAL VARIANTS CHARACTERIZATION GENERAL
- Cutaneous Vasculitis Update: Diagnostic Criteria, Classification, Epidemiology, Etiology, Pathogenesis, Evaluation and Prognosis.
Carlson JA, Ng BT, Chen KR.
From the *Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, New York; daggerDepartment of Dermatology, Saiseikai Central Hospital, Tokyo, Japan; and double daggerDepartment of Pathology, Albany Medical College, Albany, New York.
Am J Dermatopathol. 2005 Dec;27(6):504-528. Abstract quote
Vasculitis, inflammation of the vessel wall, can result in mural destruction with hemorrhage, aneurysm formation, and infarction, or intimal-medial hyperplasia and subsequent stenosis leading to tissue ischemia. The skin, in part due to its large vascular bed, exposure to cold temperatures, and frequent presence of stasis, is involved in many distinct as well as un-named vasculitic syndromes that vary from localized and self-limited to generalized and life-threatening with multi-organ disease.
To exclude mimics of vasculitis, diagnosis of cutaneous vasculitis requires biopsy confirmation where its acute signs (fibrinoid necrosis), chronic signs (endarteritis obliterans), or past signs (acellular scar of healed arteritis) must be recognized and presence of extravascular findings such as patterned fibrosis or collagenolytic granulomas noted. Although vasculitis can be classified by etiology, many cases have no identifiable cause, and a single etiologic agent can elicit several distinct clinicopathologic expressions of vasculitis.
Therefore, the classification of cutaneous vasculitis is best approached morphologically by determining vessel size and principal inflammatory response. These histologic patterns roughly correlate with pathogenic mechanisms that, when coupled with direct immunofluorescent examination, anti-neutrophil cytoplasmic antibody (ANCA) status, and findings from work-up for systemic disease, allow for specific diagnosis, and ultimately, more effective therapy.
Herein, we review cutaneous vasculitis focusing on diagnostic criteria, classification, epidemiology, etiology, pathogenesis, and evaluation of the cutaneous vasculitis patient.VARIANTS EDEMATOUS SCARRING VASCULITIC PANNICULITIS Edematous, scarring vasculitic panniculitis: a new multisystemic disease with malignant potential.
Ruiz-Maldonado R, Parrilla FM, Orozco-Covarrubias ML, Ridaura C, Tamayo Sanchez L, Duran McKinster C.
Department of Pediatric Dermatology, National Institute of Pediatrics, Mexico City, Mexico.
J Am Acad Dermatol 1995 Jan;32(1):37-44 Abstract quote
BACKGROUND: Hydroa vacciniforme (HV) is a disease of unknown origin characterized by erythema, vesicles, necrosis, and varicelliform scars in light-exposed skin. Systemic involvement is absent. A few patients have been reported with "severe HV" with systemic involvement, development of non-Hodgkin's lymphoma, and a poor prognosis.
OBJECTIVE: Our purpose was to characterize and differentiate our patients' disease from HV.
METHODS: We performed a retrospective clinicopathologic study of 14 children previously diagnosed as having "severe HV."
RESULTS: The extension and severity of the cutaneous lesions, fever, wasting, failure to thrive, hepatosplenomegaly, vasculitis, panniculitis, and potential development of lymphoma are features that clearly differentiate edematous scarring vasculitic panniculitis from HV.
CONCLUSION: Edematous scarring vasculitic panniculitis is a novel multisystemic disease with malignant potential that is not related to classic HV.
LYMPHOCYTIC VASCULOPATHY
A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease.
Oh CW, Lee SH, Heo EP.
Department of Dermatology, College of Medicine, GyeongSang National University, South Korea.
Am J Dermatopathol. 2003 Oct;25(5):423-7 Abstract quote.
A previously healthy 14-year-old Korean male presented with striking hemorrhagic acral livedo associated with anti-ribonucleoprotein antibodies. His skin biopsy revealed a striking lymphocytic vascular reaction with mild superficial perivascular lymphocytic infiltrates, perivascular extravasation of red blood cells, and leukocytoclastic debris. Lymphocytic vasculitis (LV) is defined in different ways by different authors. The above biopsy findings do not fulfill the criteria for LV of all authors.
Chronic lymphocyte-mediated endothelial cell injury may play an important role in the pathogenesis of collagen vascular disease. Cell-mediated cytotoxicity against endothelial cells or other vessel wall components is thought to be the possible pathologic mechanism of LV.
We present a patient with early undifferentiated connective tissue disease (EUCTD) with hemorrhagic acral livedo, who finally developed systemic lupus erythematosus. Even though the histopathologic findings do not meet the most rigorous definition of LV, LV may a presenting sign of EUCTD. The purpose of this report is to suggest reconsideration of the diagnostic criteria of lymphocytic vasculitis.
Immunoglobulin A-associated lymphocytic vasculopathy: a clinicopathologic study of eight patients.Crowson AN, Magro CM, Usmani A, McNutt NS.
Central Medical Laboratories, Winnipeg, Manitoba, Canada, Department of Dermatology, University of Oklahoma, OH, USA, Regional Medical Laboratories, St John's Medical Center, Tulsa, OK, USA, Department of Pathology, Division of Dermatopathology, Ohio State University, Columbus, OH, USA, and Department of Pathology, Division of Dermatopathology, Weill Medical College of Cornell University, New York, NY, USA.
J Cutan Pathol 2002 Nov;29(10):596-601 Abstract quote INTRODUCTION: Cutaneous IgA-associated vasculitis can be a clue to Henoch-Schonlein purpura (HSP), which typically comprises renal and gastrointestinal tract disease and arthritis, whereby prominent and predominant IgA deposits within the cutaneous vasculature provoke a pustular leukocytoclastic vasculitis.
DESIGN: We describe eight patients with a novel expression of a cutaneous IgA vascular injury syndrome, namely a lymphoid vasculopathy which clinically and light microscopically resembled a pigmentary purpura (PP) in six, and correlate direct immunofluorescence (DIF) and clinical features to light microscopy.
RESULTS: Among associated diseases were prior viral infection, an HSP symptom complex, an undifferentiated connective tissue disease syndrome, lupus erythematosus profundus (LEP), Degos' disease and Berger's disease. Skin lesions comprised non-palpable petechial lesions involving lower extremities in all cases and also the upper extremities in two. A superficial perivascular lymphocytic infiltrate unaccompanied by vascular fibrin deposits was associated with prominent erythrocyte extravasation including into the epidermis. Mural and extravascular fibrin deposition was seen in one biopsy from a PP-like lesion and mural fibrinoid necrosis was seen in the cases of LEP and Degos' disease; in biopsies from these three cases, the presence of fibrin deposition warranted use of the appellation 'lymphocytic vasculitis'. In all patients, DIF showed prominent and predominant IgA deposits.
CONCLUSIONS: A non-necrotizing lymphocytic purpuric vascular reaction is one manifestation of vascular IgA deposition in the skin. A subpopulation of human lymphocytes bear surface Fc receptor and/or C3 receptors ('complement receptor lymphocytes') which can bind circulating immune complexes (ICs) or C3 generated via activation of the alternative complement cascade. Thus, circulating ICs are a potential pathogenic basis of this eruption, the histologic differential diagnosis of which is idiopathic PP and PP of drug or viral etiology.
REACTIVE ANGIOMATOSIS
Cutaneous reactive angiomatoses: Patterns and classification of reactive vascular proliferation.
Rongioletti F, Rebora A.
J Am Acad Dermatol. 2003 Nov;49(5):887-96. Abstract quote
New and relatively old types of vascular (capillary) proliferations in the skin have been described or better categorized in the last few years.
They include reactive angioendotheliomatosis, acroangiodermatitis (pseudo-Kaposi sarcoma), diffuse dermal angiomatosis, intravascular histocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis (angiomatosis with cryoproteins). Clinically, all of them present with multiple, erythematous-violaceous and purpuric patches and plaques, sometimes evolving toward necrosis and ulceration with a wide distribution but a propensity to involve limbs.
Histologically, they are characterized by different patterns of intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, pericytes, and sometimes histiocytes. Although these angioproliferations can histologically mimic vascular tumors, they are reactive in that they seem to originate from the occlusion of vascular lumina by different localized or systemic disorders and the vascular proliferation stops after the inducing hypoxic stimulus has been withdrawn.
In this article, the authors review all these forms of reactive angioproliferations in the skin, suggest a novel title, cutaneous reactive angiomatoses, and propose a unifying pathogenetic mechanism.SKELETAL MUSCLE VASCULITIS
Skeletal muscle vasculitis exclusive of inflammatory myopathic conditions: A clinicopathologic study of 40 patients.Prayson RA.
Department of Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, OH.
Hum Pathol 2002 Oct;33(10):989-95 Abstract quote Vasculitis is an infrequently encountered pathology in skeletal muscle biopsy specimens, the diagnosis of which has significant therapeutic implications.
This study retrospectively reviewed the clinicopathologic features of 40 patients with vasculitis (infiltration of vessel walls by inflammatory cells) diagnosed via skeletal muscle biopsy during a 27-year period of time. Cases of vasculitis associated with inflammatory myopathic conditions, such as polymyositis or dermatomyositis, were excluded from study. Forty patients, including 21 women and 19 men ranging in age from 19 to 83 years (mean of 52 years), formed the study group.
The most common clinical presentations included muscle pain (n = 22, 55%), paresthesias (n = 16, 40%), and weight loss (n = 15, 38%). Westergren erythrocyte sedimentation rate was known in 31 patients and ranged from 12 to >150 mm/h (mean, 61 mm/h). Necrotizing vasculitis was identified in 35 patients (88%). The vasculitic process involved multiple vessels in 30 cases (75%). Vessel wall scarring and/or fibrosis were identified in 11 patients (28%), thrombi in 3 patients (8%), eosinophils in 2 patients (5%), and granulomas in 1 patient (3%). Neurogenic changes were observed in the majority of biopsy specimens as follows: angular atrophic esterase-positive muscle fibers (n = 35, 88%), grouped atrophy (n = 14, 35%), fiber type grouping (n = 17, 43%), and target fibers (n = 6, 15%). Other commonly identified pathologies included scattered degenerating muscle fibers (n = 15, 38%), regenerating muscle fibers (n = 17, 43%), and type II muscle fiber atrophy (n = 10, 25%). Thirty-three patients also underwent sural nerve biopsy. Vasculitis was identified in the nerve in 26 of 33 of these patients (79%). Peripheral nerve vasculitis was classified as necrotizing in 15 cases (57.7%) and as nonnecrotizing in 11 cases (42.5%). Seventeen of 33 patients (52%) with known follow-up showed clinical improvement with steroid/immunosuppressive therapy.
The following 2 conclusions were made: the majority of vasculitis cases arising outside the setting of inflammatory myopathy in skeletal muscle are necrotizing, and the predominant muscle pathology in the setting of vasculitis is neurogenic atrophy, likely caused by concomitant involvement of the peripheral nervous system.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES PSEUDOVASCULITIS, CUTANEOUS
From the Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, NY; and the Department of Dermatology, Saiseikai Central Hospital, Tokyo, Japan.
Cutaneous pseudovasculitis represents a heterogeneous collection of disorders that are capable of simulating cutaneous vasculitis and can be broadly classified into diseases that produce hemorrhage (petechiae, purpura, and ecchymoses) or vessel occlusion with resultant livedo, cyanosis, ulcers, digital necrosis, and/or gangrene. Overlap is not uncommon, but if present, one mechanism dominates.
Hemorrhagic pseudovasculitis is due to vessel wall dysfunction (incompetence), which can be related to diverse factors that include vessel wall deposition of metabolic substances (amyloid, calcium), nutritional deficiencies (scurvy), nonvasculitic inflammatory purpura (pigmented purpuric dermatitis, arthropod, viral and drug reactions), degeneration of the vessel wall and supporting stroma (senile/solar purpura), direct vessel wall invasion of infective organisms, coagulation-fibrinolytic disorders (eg, thrombocytopenia), and vessel wall trauma. Cyanotic-infarctive pseudovasculitis is due vaso-occlusion by emboli, thrombi, or fibrointimal hyperplasia (endarteritis obliterans) and includes varied conditions such as purpura fulminans, Coumadin necrosis, antiphospholipid antibody syndrome, cardiac myxoma, cholesterol embolization, calciphylaxis, and radiation arteritis.
Delayed and inappropriate diagnosis of pseudovasculitis leads to incorrect management and exposure to potentially deleterious treatment modalities such as corticosteroids and cytotoxic agents. The diagnosis of a pseudovasculitic disorder requires a high index of suspicion and should always be part of the differential diagnosis of vasculitis.
Skin biopsy is a crucial step in differentiating pseudovasculitis from authentic vasculitis; absence of histologic evidence of vasculitis, particularly after multiple biopsies, should direct evaluation and diagnosis towards pseudovasculitis.Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fifth Edition. Mosby Elesevier 2008
Elastin-Specialized elastic fibers present within the wall of larger vessels giving it pliability. An elastin stain or VVG stain is often used by the pathologist to highlight the fibers.
Fibrinoid Necrosis-Necrosis of the small vessel wall with deposition of fibrin. A hallmark of leukocytoclastic vasculitis.
Nuclear Dust-Breakdown products of the nuclei of inflammatory cells, usually neutrophils. Often present with leukocytoclastic vasculitis.
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